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1.0 Introduction
The cause of vaso-occlusive crisis (VOC) is believed to be ischemic tissue injury from the obstruction of blood flow by
sickled erythrocytes. Reduced blood flow causes hypoxia and acidosis. This further increases the sickling process,
leading to further hypoxia and acidosis—a cycle that eventually leads to ischemic tissue injury. Each VOC varies in
intensity and duration. Infection, fever, acidosis, hypoxia, dehydration, sleep apnea, and exposure to extremes of heat
and cold can precipitate crises. Often, no cause is identified.
Painful VOC is the most frequent complication of Sickle Cell Disease. Common sites of pain include bone (extremities,
dactylitis or hand/foot syndrome, back) and abdominal pain. Bone pain, the most common type of VOC, may or may not
be accompanied by swelling, low-grade fever, redness, and warmth. It may be symmetrical, asymmetrical, or migratory.
Dactylitis is a common presentation in infants and toddlers; back and abdominal pain are more common in older children.
Abdominal pain in children with sickle cell disease is usually a simple VOC, but other diagnoses may present similarly
(splenic sequestration, liver sequestration, appendicitis, pancreatitis, biliary colic and cholecystitis, urinary tract infection,
pelvic inflammatory disease, etc.) and should be ruled out. In addition, pneumonia and chest crisis may present as, or
accompany abdominal pain. During a severe painful crisis, a patient may also develop an acute chest syndrome, or a
CNS event.
Pain should be treated early and aggressively. No laboratory features are pathognomonic of VOC; diagnosis is based
strictly on the history and physical examination. When treating a painful crisis, the Healthcare Provider needs to be aware
that concurrent illnesses such as an acute sequestration, priapism, aplastic episode, or fever/sepsis (see other protocols)
may also occur, which must be dealt with concurrently.
This clinical practice guideline has been developed for the management of sickle cell patients with an acute painful
episode who present to the emergency department and/or inpatient units.
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 1 of 9
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electronic SharePoint version prior to use.
A Recommendation supported by at least one randomized controlled trial, systematic review or meta-
analysis.
B Recommendation supported by at least one cohort comparison, case study or other experimental study.
2.1.1 Place patients with concurrent fever of 38.5°C oral (38°C axilla) or higher immediately into a room, and see
Fever: Guideline for Management in Children with Sickle Cell Disease ==> . Do not delay administration of
empiric antibiotics.
The threshold noted above is slightly higher than the normal definition of fever given that a low-grade fever
may be secondary to VOC in the absence of any bacterial infection. Children who look unwell and have signs
suggestive of sepsis require bloodwork and antibiotics.
2.1.2 For acute chest syndrome refer to and follow other guidelines or sickle cell management protocols:
Acute Chest Syndrome ==>
2.1.3 Place children without fever in a room as soon as possible (based on CTAS score), and conduct a brief
history and physical concurrently with other measures including pain assessment and initial treatment ideally
within one hour of registration (or 30 minutes from triage).
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 2 of 9
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
2.1.4 In the presence of chest pain or respiratory symptoms request a chest x-ray and monitor the child’s O2
saturation. If the patient has severe respiratory distress, test venous blood gas (VBG) levels as well (refer to
Acute Chest Syndrome CPG).
2.1.5 Insert an IV if the patient is dehydrated or in moderate to severe pain not responding to adequate oral
treatment. Start cardiopulmonary monitoring and obtain a CBC, differential and reticulocyte count. Serum
electrolytes (Na, K, glucose, urea, creatinine) should be ordered prior to IV fluid administration as per the
hospital’s Fluid and Electrolyte Administration Clinical Practice Guideline.
2.1.6 Notify the Haematology Consult Service to see patients with any of the following:
hemodynamic instability
sepsis
meningitis
stroke, aplastic crisis, or acute splenic sequestration
O2 saturation <90% on room air
priapism
or otherwise concerned about the patient’s clinical status
2.1.7 Consider physical and psychological interventions such as heating pads, massage, warm baths, and other
comfort measures. The Child Life Specialist can recommend structured activity. Imagery and distraction are
helpful.
2.1.8 Children with sickle cell disease who have a painful episode WITHOUT fever, breathing difficulties or other
medical problems may be admitted to the Sickle Cell Day Hospital between 0800 and 1400, Monday through
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 3 of 9
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electronic SharePoint version prior to use.
Friday (refer to the Sickle Cell Pain Day Hospital guideline). The last admission will be no later than 1400.
Page the Sickle Cell Pain Pager at (416) 530-7155.
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 4 of 9
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 5 of 9
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
2.2.1 Hospitalization is indicated if pain control with oral analgesics is not adequate, or if concerns relating to fever,
dehydration, etc.
2.2.2 Patients to be admitted to units 7BCDE under Paediatric Medicine. Stable patients may be transferred for
admission to a Satellite Sickle Cell Centre – Brampton Civic Hospital or Rouge Valley Centenary.
2.2.3 Observe patients for signs of deterioration through continuous cardiac and O2 saturation monitoring with
continuous opioid infusions. When continuous monitoring is no longer required, vital signs assessed as per
BedsidePEWS, fluid input and output, and daily weight. Assess the child’s comfort level q4h, and before and
after each pain medication and non-pharmacologic intervention, with a consistent pain tool. See Pain
Assessment Policy ==>
2.2.4 Unless there are contraindications, initiation of incentive spirometry and referral to physiotherapy is indicated
for children admitted with chest, back, neck or abdominal VOC. For patients with limb/other VOC, a
physiotherapy consult may still be warranted. The physiotherapist will encourage ambulation and activity
within the child’s tolerance. Other health professionals should promote mobility as per physiotherapy
recommendations. Note that nursing may initiate incentive spirometry prior to assessment by physiotherapy.
See Cardiopulmonary Physiotherapy policy.
2.2.5 Consider physical and psychological interventions such as heating pads, massage, warm baths, and other
comfort measures. The Child Life Specialist can recommend structured daily activity. Imagery and distraction
are helpful.
2.2.6 If pain is not managed despite appropriate analgesics consult Acute Pain Services.
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 6 of 9
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
Acetaminophen and Acetaminophen AND an NSAID are recommended unless contraindications exist.
NSAID
Choice of recommended
NSAIDs include:
Ibuprofen
Naproxen
Ketorolac
2.2.8 For a pain crisis in children 8 years of age and older, consider a Patient Controlled Analgesia (PCA) pump. As
well, consider a PCA pump for patients previously treated with a PCA pump or for patients not achieving
adequate analgesia. A PCA can be obtained by paging the Acute Pain Services at (416) 235-8912, who will
set the PCA’s basal rate, bolus rate, and lockout time.
2.2.10 Hydration: Continue IV/PO fluids for a total fluid intake (TFI) of maintenance.
2.2.11 Oxygen: Studies do not support systematic use of oxygen for VOC. However, hypoxia may occur in children
with VOC, resulting in increased sickling. Therefore, monitor O2 saturation regularly, and provide oxygen to
patients exhibiting hypoxemia (<95%).
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 7 of 9
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
Revised (2011)
1. Melanie Kirby, MD, Staff Physician, Division of Haematology/Oncology
2. Jeremy Friedman, MD, Head, Division of Paediatric Medicine
3. Isaac Odame, MD, Staff Haematologist
4. Upton Allen, MD Chief, Division of Infectious Diseases
5. Dennis Scolnick MD, Staff Physician, Division of Emergency Medicine
6. Lori Palozzi RN, NP Nurse Practitioner Acute Pain Service
4.0 References
1. Baskin MN, Goh XL, Heeney MM, Harper, MB. Bacteremia risk and outpatient management of febrile patients
with sickle cell disease. Pediatrics. 2013;131(6):1035-1041.
2. Bellet PS, Kalinyak KA, Shukla R, Gelfand MJ, Rucknagel DL. Incentive spirometry to prevent acute pulmonary
complications in sickle cell diseases. N Engl J Med. 1995;333(11):699–703.
3. Chang TP, Kriengsoontorkij W, Chan LS, Wang VJ. Predictors for bacteremia in febrile sickle cell disease children
in the post-7-valent pneumococcal conjugate vaccine era. Journal of Pediatric Hematology Oncology. 2013;35
(5):377-382.
4. Griffen TC, McIntire D, Buchanan GR. High-dose intravenous methylprednisolone therapy for pain in children and
adolescents with sickle cell disease. N Engl J Med. 1994;330(11):733–37.
5. Jacobson SJ, Kopecky EA, Joshi P, Babul N. Randomised trial of oral morphine for painful episodes of sickle-cell
disease in children. Lancet. 1977;350:1358–61.
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 8 of 9
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
6. National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: Expert panel
report, 2014. www.nhlbi.nih.gov/sites/www.nhlbi.nih.gov/files/sickle-cell-disease-report.pdf. Accessed on May 18,
2015
7. Reid CD, Charache S, Lubin B (eds). Management and Therapy of Sickle Cell Disease, 3rd edition. National
Institutes of Health Publication No 95-2117, Bethesda, Maryland, 1995.
8. Robieux IC, Kellner JD, Coppes MJ, Shaw D, Brown E, Good C, O’Brodovich H, Manson D, Olivieri NF, Zipursky
A, Koren G. Analgesia in children with sickle cell crisis: comparison of intermittent opioids vs. continuous
intravenous infusion of morphine and placebo-controlled study of oxygen inhalation. Pediatr Hematol Oncol.
1992;9:317–26.
9. Savlov D, Beck CE, DeGroot, J, Odame I, Friedman JN. Predictors of bacteremia among children with sickle cell
disease presenting with fever. Pediatr Hematol Oncol. 2014;36(5):384-388.
10. Shapiro B. The management of pain in sickle cell disease. Pediatr Clin North Am. 1989;36:1029–45
Attachments:
Revision History.docx
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 9 of 9